by Wendy L. Marshall

My sister, Rorie Susan Buchanan, died in the middle of the afternoon on Oct. 14, 1997, of Amyotrophic Lateral Sclerosis (ALS) — also known as Lou Gehrig’s disease — a fatal neurological disorder that causes a progressive degeneration of the motor cells in the spinal cord and brain. Unable to function normally, the motor neurons in the brain stop sending the impulses that are required for the muscles to work so they atrophy, especially those in the arms and legs, and those that involve speech, swallowing and breathing.

Lying motionless in a hospital bed in her living room in Three Rivers, Mich., morphine tricking her brain into thinking her body was not in distress, Rorie had been semi-conscious for at least a day. Her husband, her children, her mother, her son- and daughter-in-law, and close friends stayed with her until she finally closed her eyes, smiled a little smile and died.

Months before, when she could still get enough air in her lungs to force it past her larynx and speak, she had confided that she was afraid that when her lungs failed, her death would be like drowning. That was her nightmare. After all she’d been through she just wanted to die peacefully, life leaving her gracefully like a dancer gliding across a ballroom floor. That image gave her comfort. She loved to dance.

When Rorie was 12 and I was 11, the P.E. teacher at Coronado Elementary School in West Covina, Calif., made us forego the usual outside activities and dance. Every Friday, with the boys lined up on one side of the cafeteria and the girls on the other, she would turn on the record player, go down the roll sheet of fifth and sixth graders, assign partners, and make us assume the proper stance for the box step. For the next 30 minutes she would shout out the cadence and the movements we were supposed to learn. “Don’t look at your feet to see if you are doing it right,” she would yell as she walked around the room making adjustments to adolescent bodies. “Just dance.”

As a kid, Rorie was a klutz. She was tall and gangly and just couldn’t seem to get her arms and legs organized into one cohesive unit. She could, and did, break her arm while fixing her hair in the bathroom – she slipped and hit the sink. She hacked a gaping hole in her shin while demonstrating the finer points of using a hand axe to eager young Camp Fire Girls at a summer day camp. Sports were of no interest to her, except as a rabid fan. Still, she loved to dance, and frankly, over the course of our growing up, she achieved some grace on the dance floor. Thirty-five years later, it was that grace her family would look for.

In the exact opposite of those PE teacher’s admonitions, her children wanted desperately to watch Rorie’s feet — every step, every shuffled inch — so that they might learn. They hoped that in the dance there would be a great lesson, a great message. They wanted her to teach them about life so they could live after her death.

But Rorie was used to being in charge of her life, in charge of her home, and her fiercely independent spirit made this new dance difficult.

3/23/97: I want her to seep into my skin, to tell me everything now, so I will always have her with me. But our relationship isn’t like that. She made it perfectly clear from the beginning that this was her disease and she would handle it her way. Decisions would be made by her and Dad. End of discussion. We kids were not included, but we all wanted something to do, something to focus on. Doesn’t she understand? Doesn’t she see that we still need her as a mom? No one will ever take her place. I feel a hole already. (Oldest daughter’s journal)

Rorie’s three kids were only in their late teens and early 20s. They watched her feet every day, and measured what life was left when her steps faltered. They struggled with an intensity of emotions they’d never experienced before because despite all evidence to the contrary, the world had shifted.

4/1/97: I remember when my mother sat me down and told me what was wrong with her. I don’t remember the next few minutes after that. All I could think of was, yeah right. This isn’t funny. Then, as I went about the next few months and the diagnosis was confirmed, I decided to pretend I didn’t care. Naturally I thought if I didn’t have to think about it, it wasn’t true. Boy, was I wrong. (Youngest daughter’s journal)

I was lucky in a way. I lived more than two thousand miles away. I didn’t have to watch her lose every shred of modesty, privacy, and dignity as the disease progressed. My mother’s sadness was palpable whenever I talked to her. She was breaking the cardinal rule of parenting: She was going to outlive her child.

4/23/77: Awakened this morning by a call from Rorie. She wanted me to do something for her and I couldn’t understand her. I was so frustrated and so angry that it reduced me to tears. It’s so hard to see her go through this. I want to do SOMETHING, anything to help her and I can’t do one thing to make it go away. (Mother’s journal)

Rorie’s children were asked to do things for their mother that children are not normally asked to do. They had to bathe, dress, feed, and tend to toilet care. They had to shave her legs, put on her makeup, brush her teeth and her hair. They were at the dinner table the first time she choked on her food. It frightened everyone. Her husband raised her right arm over her head and pounded on her back like he used to do when the kids were small. The rest of us jumped up and away from the table, not knowing what to do and not knowing what to say after the episode was over.

7/3/97: How do you look your sister in the eye and joke about something as small as a kernel of rice taking away what little breath she has? I saw the fear in her eyes and she saw it in mine.

No one knows what causes ALS, but it usually strikes adults between the ages of 40 and 70. Unfortunately, there have been plenty of people who have died in their 20s and 30s, as in Gehrig’s case. Once thought rare, between two and seven of every 100,000 people in the United States are diagnosed with ALS. According to the United States National Institutes of Health, more than 5,000 new cases are identified every year – about 14 cases a day. The ALS Association now estimates that some 300,000 men and women who are alive and apparently well today will eventually die of the disease.

Gehrig, first baseman for the New York Yankees was called the “Iron Man” because he set a major league record of 2,130 consecutive games. His last game in the streak was on April 30, 1939, after which he told Yankees’ manager, Joe McCarthy, to take him out of the lineup. By doing so, Gehrig ended the streak that he began June 1, 1925. He was in a slump and he felt he was hurting the team. ALS took him out of the game he loved.

It took Rorie out of her game too.

It was Feb. 20, 1995, when Rorie was essentially taken out of the lineup. She was certainly never an athlete. She could barely bounce a ball. She was a daughter, a sister, a wife, a mother, a grandmother, a secretary, a bookkeeper, a hostess of the annual Christmas House circuit in her little town, a member of the bell choir at her church, a neighbor, a friend.

7/5/97: My sister has a disease named after a New York Yankee. Unbelievable. She doesn’t even like baseball. Football is her passion. She’s a hardcore Denver Bronco fan. What about a second opinion? A third if she has to. Screw the insurance company. How did she get this? Will I get this? Will anybody else in the family get this? Tell me you have breast cancer. I know what that is. I would know what to do, what questions to ask, and where to go to ask them. There would be chemo. You might lose your breasts, your hair, your appetite, your sense of taste and smell, but you’d have a chance. You’d crawl through hell, but at least you’d have a chance. There’s no chance with this. None.

Jean-Martin Charcot, a French neurologist, first identified ALS in 1869. Although they vary with each individual, early symptoms include frequently dropping things, tripping, slurred speech, muscle cramps and twitches, abnormal fatigue of the arms and/or legs and uncontrollable periods of laughing or crying. The symptoms usually manifest themselves first in the hands or the feet, making routine daily chores difficult. As the weakening and paralysis spread to the muscles of the trunk, ALS eventually affects swallowing, chewing and breathing.

The sick irony is that although it ravages the body, the disease leaves the mind and the senses intact. Patients can feel an ant crawling across their arm. They just can’t lift their arm and shoo it away.

8/14/97: It would be easier if she wasn’t aware of everything I do and say, and the way I do it and say it. She was the one who told me that you could say anything you want to a baby as long as you say it with a smile. My grandmother tells me to be gentle. How? I don’t feel gentle. I don’t know what I feel. Anger? Fear? All of that! Everybody has shared more of my mother’s life that I have. She’ll never see me graduate from college, or get married. She won’t see my children. All she sees is the anger because right now, that’s all I have. Lou Gehrig died too long ago. Why the hell hasn’t somebody more recently famous gotten this disease? Then there would be somebody and all his friends thumping their chest screaming, “Don’t let him die. We have to find a cure.” Well, I’m thumping, and I’m screaming. “Don’t let my mom die.” Is anybody listening?

Fifty-eight years have passed since Gehrig died and doctors still don’t know what causes ALS. There is still no one conclusive test, so it becomes a long process of elimination – determining what a patient doesn’t have – in order to tell them what they do. There is still no treatment or cure. There is no federally funded research, no poster child, no telethon, and no voice.

In 1993 researchers identified a defective gene on Chromosome 21 as the potential cause of an inherited form of the disease, familial ALS; but only a small percentage of the cases diagnosed in the United States are considered to be hereditary. Typically, cases are classified as sporadic ALS, which is the form of the disease that Rorie had. Earlier research by Johns Hopkins University Hospital proposed that glutamate, an amino acid responsible for carrying messages between brain cells, may be involved. After it has done its work in the brain, the glutamate is supposed to be reabsorbed by a special protein called a transporter. ALS researchers suspect that a defective transporter, unable to absorb enough glutamate, destroys the motor neurons in the brain. Still, more research is needed to test the validity of the Johns Hopkins theory and to see if drugs can be developed to address the glutamate absorption problem.

9/7/97: So now my sister has a bad gene? She didn’t know it. None of us did. You couldn’t miss the obvious manifestations of her gene pool – a head-full of thick, auburn hair, dark brown eyes, and delicate ivory skin splattered with thousands of tiny freckles. I guess the bad gene was there too, or so the researchers say, lurking inside her 5 ft. 11 ½ inch frame on Chromosome 21. Unfortunately, that little speck on Rorie’s DNA spiral was a fatal flaw. Makes you wonder how any of us survive.

Looking at Rorie’s feet was her family’s foundation. As long as she was still leading the dance, they only had to follow. The thought of guiding her across the floor was terrifying. They knew it was coming. We all did. But no one wanted that role. Her family members yelled at each other out of frustration and exhaustion. They said things hoping the anger would keep the ALS at bay and keep Rorie on her feet. They wanted her to dance and to be alive; to be conscious once more of her chest expanding with a lungful of air.

12/25/97: I miss her. I miss her. I miss her. Every day. All the time. I hear a song . . . it hurts. Everything somehow ties in to the pain of her leaving. I don’t feel her here anymore, and no one told me how lonely that would leave me. I keep waiting to feel her here with me. I want it to be over neatly and I never want to forget her. Now there’s a paradox. There isn’t a beginning to the feelings and emotions and there isn’t an end. I fight for an answer. I fight the hurt. Right now I’m losing. I want a hug from my mom. Mom knows what to do and to say to let me cry. Mom knows how to make me feel better. (Son’s journal).

I was at work when Rorie died, but before I got home to get the telephone call from my mother, I received an e-mail message from a cousin.

Dear gang: I had a very fleeting image this afternoon of a smiling Rorie on her way. I could have sworn she was dancing.

Wendy L. Marshall is a journalist and author of the nonfiction book William Beaudine: From Silents to Television. She is currently working on a novel.